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Advances in Vasculitis: Notes From the Field
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The Cover Story of this issue of Healio Rheumatology is dedicated to vasculitis. I have been looking forward to writing this Editorial for a number of reasons, not least of which is the fact that I have spent a large part of my career seeing patients with various forms of vasculitis and have had a “bird’s eye view” of the maturation of the field from an area of complex curiosity to highly productive basic, translational and clinical research. The discussions with many leading figures in the field on this issue testify to this deserved status. Not long ago, guidance on the diagnosis and management of patients with these uncommon disorders came from a dedicated few who made it their business to bring these diseases into the light. Today, however, we are guided by increasingly robust evidence and it is a challenge for general rheumatologists to keep up with the pace of discovery.
While this is not an historical piece, I feel obliged to provide some context by recognizing some important people who enabled the field. Central to this journey are the contributions by investigators from the NIH – notably, the early work of Sheldon M. Wolfe, MD; and Anthony S. Fauci, MD, whose contributions were based on methodical observation and method. Their careful and insightful work first informed us that the previously thought incurable and life threatening diseases of Wegener’s granulomatosis and polyarteritis nodosa could be successfully managed with cyclophosphamide and glucocorticoids. The next phase of their work was led by my colleague Gary S. Hoffman, MD; who dedicated his career to refining this initial work and developing ways to make therapies safer and more effective for patients.
Into the Mainstream
© 2017 Shutterstock.com/Venera Salman
The real pivot point in the field occurred when Hoffman brought together numerous individuals working in the area to form a collaborative network known as the International Network for the Study of Systemic Vasculitidies (INSSYS). This effort brought the field of vasculitis into the mainstream and demonstrated that multicenter trials were not only possible, but also could potentially have great impact.
Building on this success, today we have the robust Vasculitis Clinical Research Consortium (VCRC), which was founded in 2003, and the highly productive European Vasculitis Study (EUVAS) group, which was founded in the early 1990s. One of the greatest contributions of these groups is the demonstration of the power of collaboration. Numerous countries have their own consortia which are also making strong contributions.
Today we look for guidelines emanating from numerous countries and professional organizations, all fed by data from multiple randomized controlled clinical trials, guiding us in the assessment and management of most forms of systemic vasculitis. These trials and guidelines have indisputably improved our ability to treat patients with vasculitis. Consider methotrexate for limited anti-neutrophil cytoplasmic antibodies (ANCA) disease, rituximab for ANCA disease, antimetabolites for limited disease and maintenance remission and, of course, the increasingly refined classification and diagnostic criteria available to us.
Greater Understanding Ahead
What about the future? As they say, it is hard to predict but we are on the verge of greater understanding in terms of pathogenic mechanisms that may provide clues to more specific and better therapies for numerous diseases. Think curing hepatitis C (HCV) on a global scale and the prospect of ending the rare but formidable HCV-associated cryoglobulinemia. Finally, consider the reality of interleukin-6-targeted therapy in the management of large vessel vasculitis, which while not yet approved has been granted breakthrough status by the FDA. More to come on this topic next month!
I would like to close by asking you to reach out to me during the next few months to let me know what topics in our field excite you and what you would like us to cover. You can email me at calabrl@ccf.org or follow me on Twitter @LCalabreseDO. Thanks for reading.
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- Leonard H. Calabrese, DO, is the Chief Medical Editor, Healio Rheumatology, and Professor of Medicine, Cleveland Clinic Lerner College of Medicine of Case Western Reserve University, and RJ Fasenmyer Chair of Clinical Immunology at the Cleveland Clinic.
Disclosure: Calabrese reports he is a consultant for Genentech, Pfizer, Bristol-Myers Squibb, GlaxoSmithKline, Sanofi, Jansen and AbbVie; and is on the speakers bureau for Genentech, AbbVie and Bristol-Myers Squibb and Crescendo Bioscience.